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Introduction: The third ventricular tumor is one of the most difficult lesions to approach. However, if a cavum septum pellucidum (CSP) and cavum vergae (CV) are present, approaching the tumor might be easier by exploiting them. Here, we describe the achievement of a favorable outcome in treating a patient with third ventricular tumor with CSP and CV using an anterior transcallosal interforniceal approach for tumor removal. Case description: A 52-year-old woman presented with acute hydrocephalus symptoms. Head imaging revealed a tumor filling the third ventricle and protruding into the right lateral ventricle through the right foramen of Monro. We also confirmed the presence of CSP and CV. After ventricular drainage, we performed a craniotomy to remove the tumor via an anterior transcallosal interforniceal approach. Almost all of the intraventricular tumor was removed easily without any complications, such as memory impairment. Conclusion: If CSP and CV are present, an anterior transcallosal interforniceal approach can be safely and effectively used to remove a third ventricular tumor.